[Jakob-Creutzfeld disease--intra vitam diagnosis?]

The diagnosis of Jakob-Creutzfeld disease was made in two women (aged 59 and 76 years) whose course was marked by rapidly progressing signs of dementia, myoclonus and rhythmic bi- and tri-phasic steep waves in the EEG. The diagnoses were confirmed neuropathologically at autopsy. Since the disease is transmissible, precautions must be taken if the disease is suspected: wearing of disposable gloves, disinfection of instruments by one-hour autoclaving at 121 degrees C, disinfection of potentially contaminated surfaces with 5% sodium hypochlorite, and avoidance of skin cuts or punctures by instruments or syringes.